脑病相关神经节苷脂研究进展

神经节苷脂是一种糖链结构上包含有唾液酸的酸性鞘糖脂,是动物细胞膜的重要组成成分,并在细胞膜表面上参与各种重要的生物学进程.正常生理情况下,脑内的神经节苷脂在神经细胞的形态稳定和神经信号的传递等生物进程中发挥至关重要的作用,这些生物进程和大脑的生长发育与认知发展密切相关.在一些患者各脑区检测到的神经节苷脂含量与种类的明显改变,提示着不同脑部疾病的发生与发展,例如在一些脱髓鞘疾病患者脑内常常伴随有神经节苷脂减少的现象.此外,定位于胞膜上的神经节苷脂还能极大地影响阿尔茨海默病等神经退行性疾病和胶质瘤等脑部肿瘤的发生和发展.以上所述的种种病症看似发病机制相去甚远,但这些脑病之间却因为神经节苷脂的联系而具有一定的共性和发病模式,例如在数年前流行于南美的寨卡病毒与常见的神经脱髓鞘疾病格林-巴利综合症均是由于自身B细胞产生的抗GQ1b神经节苷脂抗体与脑内神经细胞膜表面GQ1b的结合所引起的.本文就脑内数种疾病涉及神经节苷脂的发病机制进行总结并概括了几种可能的共同发病模式,以期未来在脑内疾病的诊断和治疗中提供一个新的思路.

Advances of Encephalopathy Associated With Gangliosides

Gangliosides are glycosphingolipids containing one or more sialic acids in the glycan chains, which are components of the cell surface of all mammalian cells and involved in various important biological processes in cell plasma membranes. Such it plays an important role in the transmission of nerve signals and keeps morphological stability of nerve cells in normal physiological conditions. Gangliosides are particularly abundant and typically more structurally complex in the mammalian brain, which are considered closely related to brain growth and cognitive development. Significant changes of ganglioside content and types in some brain regions may herald different brain diseases occurrence and development. For example, some demyelinating diseases are associated with a significant decrease of gangliosides in brain. On the other hand, gangliosides located on the cell membrane can greatly affect the development of neurodegenerative diseases and brain tumors, such as Alzheimer"s disease and glioma. The brain diseases seem to have different pathogenesis, however, these brain diseases have certain relevance in their pathogenesis due to the existing gangliosides, such Zika fever is similar to Guillain-Barré syndrome (a common neurodemyelination disease). This paper summarizes possible common patterns of the pathogenesis of several encephalopathies associated with gangliosides, which could provide a new idea for the diagnosis and treatment of the encephalopathies.